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1.
Clin Transplant ; 38(3): e15272, 2024 03.
Article En | MEDLINE | ID: mdl-38445550

COVID-19 is a heterogenous infection-asymptomatic to fatal. While the course of pediatric COVID-19 infections is usually mild or even asymptomatic, individuals after adult heart transplantation are at high risk of a severe infection. We conducted a retrospective, multicenter survey of 16 pediatric heart transplant centers in Germany, Austria and Switzerland to evaluate the risk of a severe COVID-19 infection after pediatric heart transplantation between 02/2020 and 06/2021. Twenty-six subjects (11 male) with a median age of 9.77 years at time of transplantation and a median of 4.65 years after transplantation suffered from COVID-19 infection. The median age at time of COVID-10 infection was 17.20 years. Fourteen subjects had an asymptomatic COVID-19 infection. The most frequent symptoms were myalgia/fatigue (n = 6), cough (n = 5), rhinitis (n = 5), and loss of taste (n = 5). Only one subject showed dyspnea. Eleven individuals needed therapy in an outpatient setting, four subjects were hospitalized. One person needed oxygen supply, none of the subjects needed non-invasive or invasive mechanical ventilation. No specific signs for graft dysfunction were found by non-invasive testing. In pediatric heart transplant subjects, COVID-19 infection was mostly asymptomatic or mild. There were no SARS-CoV-2 associated myocardial dysfunction in heart transplant individuals.


COVID-19 , Heart Transplantation , Adult , Humans , Male , Child , Adolescent , COVID-19/epidemiology , Austria/epidemiology , Switzerland/epidemiology , Retrospective Studies , Heart Transplantation/adverse effects , Germany/epidemiology
2.
ESC Heart Fail ; 11(3): 1602-1611, 2024 Jun.
Article En | MEDLINE | ID: mdl-38378979

AIMS: The aim of this study is to evaluate and compare the impact of the bicaval technique versus the biatrial technique (by Lower and Shumway) in paediatric heart transplant patients. Only a few studies investigate this matter regarding the long-term outcome after paediatric heart transplantation. We compared the two surgical methods regarding survival, the necessity of pacemaker implantation. METHODS AND RESULTS: All 134 patients (aged <18 years) - (group-1) biatrial (n = 84), versus (group-2) bicaval (n = 50), who underwent heart transplantation between October 1988 and December 2021, were analysed. Freedom from events were estimated using the Kaplan-Meier method. Potential differences were analysed using the log rank test and Cox proportional hazard models. Mean ± standard deviation: Bypass time (per minutes) was higher in the group 1 as compared with group 2 (P = 0.050). Survival was not significantly different (P = 0.604) in either groups. Eighteen patients required permanent pacemaker implantation in the group 1 and only one patient required it in the group 2 (P = 0.001). CONCLUSIONS: Paediatric heart transplantation using bicaval technique results similar long-term survival compared with the biatrial technique. The incidence of atrial rhythm disorders was significantly higher in the biatrial group, requiring a higher frequency of pacemaker implantation in this group. As a results, the bicaval technique has replaced the biatrial technique in our centre.


Heart Transplantation , Humans , Heart Transplantation/methods , Male , Female , Child , Retrospective Studies , Follow-Up Studies , Time Factors , Child, Preschool , Adolescent , Survival Rate/trends , Treatment Outcome , Infant , Postoperative Complications/epidemiology
3.
Transplant Proc ; 55(7): 1656-1663, 2023 Sep.
Article En | MEDLINE | ID: mdl-37438194

BACKGROUND: Medication adherence is essential for long-term success after pediatric organ transplantation. Causes of reduced adherence should be detected early to improve the consequent medication intake. We describe the influence of switching from tacrolimus twice daily (tacrolimus-BID) to tacrolimus once daily (tacrolimus-QD) on medication satisfaction and medication adherence in patients after pediatric heart transplantation. METHODS: A retrospective analysis was conducted regarding patient satisfaction and adherence to the immunosuppressant tacrolimus after pediatric heart transplantation, before and after conversion from tacrolimus-BID to tacrolimus-QD, using questionnaires. RESULTS: Thirty-eight patients were enrolled (tacrolimus-BID: n = 35, mean age 15.7 ± 5.2 years; tacrolimus-QD: n = 38, mean age 16.2 ± 5.6 years). The amount of unadministered medication in the last 3 months did not differ between the 2 pharmaceutical forms. However, 17% (n = 6) reported unstable tacrolimus trough levels when taking tacrolimus-BID, vs 8% (n = 3) under tacrolimus-QD (P = .453). However, there was no statistically significant difference in the stability of the last 6 trough levels of each patient (P = .074). A total of 57% (n = 20) of patients had subjective side effects before conversion, compared to only 29% (n = 11) after conversion (P = .013). Regarding the intensity of the side effects, 6 patients reported strong/very strong side effects when taking tacrolimus-BID vs 1 patient when taking tacrolimus-QD (P = .250). In addition, the overall satisfaction with the immunosuppressant was higher under tacrolimus-QD (92% vs 83%; P = .508). However, this improvement was statistically not significant and may not be clinically relevant. CONCLUSIONS: The amount of forgotten medication was not reduced after conversion from tacrolimus-BID to tacrolimus-QD. However, subjective side effects as well as patient satisfaction improved under tacrolimus-QD.


Heart Transplantation , Tacrolimus , Humans , Child , Adolescent , Young Adult , Adult , Tacrolimus/therapeutic use , Retrospective Studies , Patient Satisfaction , Drug Administration Schedule , Immunosuppressive Agents/therapeutic use , Heart Transplantation/adverse effects , Personal Satisfaction
4.
Cardiovasc Diagn Ther ; 13(3): 453-464, 2023 Jun 30.
Article En | MEDLINE | ID: mdl-37405017

Background: Pulmonary hypertension (PH) is diagnosed based on an invasive evaluation of the mean pulmonary artery (PA) pressure. The morphological assessment of the pulmonary arteries was only recently not feasible. With the advent of optical coherence tomography (OCT)-imaging, an accessible tool allows to study PA morphology longitudinally. The primary hypothesis was that OCT distincts the PA structure of PH patients from control subjects. The secondary hypothesis was that PA wall thickness (WT) correlates with the progression of PH. Methods: This is a retrospective monocentric study of 28 paediatric patients with (PH group) and without PH (control group) who had undergone cardiac catheterisation including OCT imaging of the PA branches. OCT parameters analysed were WT and the quotient of WT and diameter (WT/DM) and those were compared between the PH group and the control group. In addition, the OCT parameters were aligned with the haemodynamic parameters to evaluate the potential of OCT as a risk factor for patients with PH. Results: WT and WT/DM in the PH group were significantly higher compared to the control group {WT: 0.150 [0.230, range (R): 0.100-0.330] vs. 0.100 [0.050, R: 0.080-0.130] mm, P<0.001; WT/DM: 0.06 [0.05] vs. 0.03 [0.01], P=0.006}. There were highly significant correlations between WT and WT/DM with the haemodynamic parameters mean pulmonary arterial pressure (mPAP) [Spearman correlation coefficient (rs) =0.702, P<0.001; rs=0.621, P<0.001], systolic pulmonary arterial pressure (sPAP) (rs=0.668, P<0.001; rs=0.658, P<0.001) and WT and pulmonary vascular resistance (PVR) (rs=0.590, P=0.02). Also, there was a significant correlation between WT and WT/DM and the risk factors quotient of mPAP and mean systemic arterial pressure (mSAP) (mPAP/mSAP) (rs=0.686, P<0.001; rs=0.644, P<0.001) and pulmonary vascular resistance index (PVRI) (rs=0.758, P=0.002; rs=0.594, P=0.02). Conclusions: OCT can detect significant differences in WT of the PA in patients with PH. Furthermore, the OCT parameters correlate significantly with haemodynamic parameters and risk factors for patients with PH. More investigations are required to evaluate to what extent the impact of OCT can contribute to the clinical care of children with PH.

5.
J Behav Ther Exp Psychiatry ; 81: 101884, 2023 12.
Article En | MEDLINE | ID: mdl-37307645

BACKGROUND AND OBJECTIVES: Cognitive theories assume an imbalance of intuitive and analytical reasoning in paranoid ideation. The argumentative theory of reasoning offers an approach on the primary function of reasoning and its flaws. It assumes social exchange as main purpose of reasoning. We applied this theory to delusion research and tested experimentally whether social exchange in the form of production and evaluation of arguments influences subsequent reflective reasoning. Additionally, we examined whether the social network and the frequency/preference for discussions are associated with distorted reflective reasoning and paranoid ideation. METHODS: 327 participants completed the Social Network Index (SNI), the Paranoia Checklist (PCL), and the Cognitive Reflection Test-2 (CRT2). Moreover, preference and frequency of discussions were assessed. In the discussion group (N = 165), participants produced arguments and evaluated counterarguments on two socially relevant topics. The control group (N = 162) watched a nature video instead. RESULTS: The discussion group showed lower distorted reflective reasoning than the control group. Discussion preference and/or frequency was associated with frequency and disturbance of paranoid thoughts, as well as overall paranoid ideation. LIMITATIONS: Due to the online format environmental factors could not be held constant and no intrasubject comparison of the CRT2 could be computed in the trial. Furthermore, the sample mainly consisted of psychology students. CONCLUSION: The results contribute to the understanding of distorted reflective reasoning and provides preliminary evidence that the argumentative theory of reasoning may offer a promising perspective for delusion research.


Paranoid Disorders , Problem Solving , Humans , Pilot Projects , Paranoid Disorders/psychology , Neuropsychological Tests
6.
Front Psychiatry ; 14: 1098019, 2023.
Article En | MEDLINE | ID: mdl-37032923

Hippocampal dysregulation might be a key pathophysiological factor for memory impairments in psychosis. Contemporary models particularly postulate that an imbalance of hippocampal glutamate and GABA leads to impaired memory and may thus serve as a therapeutic target to improve memory deficits. However, currently available interventions in early stages of psychosis do not explicitly target hippocampal pathology. A novel approach for manipulating hippocampus-dependent memory processes is provided via the gut microbiota. In this perspective article, we first recapitulate compelling evidence for emerging hippocampus pathology during the development of psychosis. The following sections emphasize the critical role of the gut microbiota in hippocampus plasticity and memory, and summarize existing evidence of gut microbiota alterations in different stages of psychosis. Finally, we propose a novel conceptual roadmap for future studies deciphering gut microbiota-hippocampus synergisms in emerging psychosis and argue that specific microbial supplementation might be promising for improving hippocampus-dependent memory deficits in early stages of psychosis.

7.
J Hypertens ; 41(4): 597-607, 2023 04 01.
Article En | MEDLINE | ID: mdl-36723480

AIMS: Increased arterial stiffness, measured as arterial pulse wave velocity (PWV) is associated with an elevated cardiovascular risk. Although noninvasive PWV measurement methods have been validated by invasive measurement, there is little such data on pediatric patients. The purpose of this study was to 'fill the gap' by validating PWV obtained by Mobil-O-Graph in children, adolescents in comparison to young adults. METHODS: Sixty patients (25 male, mean age 16.6 years; range 3-35 years) were included in this study. Fifty-one patients underwent cardiac catheterization after a heart transplantation (HTX) and nine for interventional atrial septal defect-closure. Specific invasive pulse wave velocities were assessed for the ascending aorta (aPWV) and entire central aorta (cPWV). These invasive PWV results were compared to simultaneously measured brachial cuff readings using Mobil-O-Graph (oPWV) stratified by age in two groups (PEDIATRICS <18 years|ADULTS ≥18 years). RESULTS: Correlation analysis showed a positive linear relation between both invasive PWV measurements and the oPWV in all ages (cPWV/oPWV: r  = 0.417, aPWV/oPWV: r  = 0.628; P  < 0.001). The oPWV data agreed better with the aPWV in mean-value comparisons and correlations with mean difference in PEDIATRICS was 0.41 ±â€Š0.41 m/s (95% confidence interval 0.27-0.55). We also found the cPWV to be faster than the aPWV particularly in adults. In addition, cPWV correlated closer with age ( r  = 0.393, P  < 0.05). CONCLUSION: Estimated oPWV using the Mobil-O-Graph demonstrated excellent accuracy in adults and pediatric patients. Therefore, the Mobil-O-Graph can be implemented as an ambulatory PWV measuring tool for pediatric cardiovascular risk stratification. CLINICAL TRIAL REGISTRATION: German clinical trial registration, DRKS00015066.


Pulse Wave Analysis , Vascular Stiffness , Adolescent , Child , Humans , Male , Young Adult , Aorta , Arteries , Blood Pressure , Oscillometry , Pulse Wave Analysis/methods
8.
Clin Transplant ; 37(3): e14710, 2023 03.
Article En | MEDLINE | ID: mdl-35576323

Tricuspid annular plane systolic excursion (TAPSE) is important in the noninvasive echocardiographic assessment of right heart function. This retrospective observational study shows correlations of TAPSE with invasive right heart catheterization parameters after pediatric heart transplantation (HTx). The study included patients after pediatric HTx with cardiac catheterizations in 2018/2019 and measurement of TAPSE (n = 52 patients with 57 examinations; 50.9% adults, 52.6% female, median age: 18.54 years). TAPSE was compared with normal values. Stepwise, linear and multiple regression were used to show influencing variables on TAPSE. Mean TAPSE z-score was -3.48 (SD: 2.25) and 68.4% of HTx-recipients showed abnormally reduced TAPSE (z-score ←2) compared to normal values. Multiple regression (p-value <0.001; corrected R2 = 0.338) showed significant correlations of time since HTx (p-value <0.001) and mPAP (p-value: 0.008) with TAPSE z-scores. Divided into subgroups (time since HTx <10 and ≥10 years), TAPSE and mPAP correlated only ≥10 years after HTx (p-value = 0.002). This study provides data of TAPSE even ≥10 years after pediatric HTx. Most patients showed a decreased TAPSE early after HTx, which improved over time. TAPSE z-scores correlated significantly with time since HTx and mPAP, especially ≥10 years post-HTx. Therefore, TAPSE must be used carefully in the early follow-up.


Heart Transplantation , Hypertension, Pulmonary , Adult , Humans , Child , Female , Adolescent , Male , Pulmonary Artery , Echocardiography , Retrospective Studies , Heart Transplantation/adverse effects , Ventricular Function, Right , Stroke Volume
9.
SSM Ment Health ; 42023 Dec 15.
Article En | MEDLINE | ID: mdl-38230394

Purpose: Despite affecting up to 20% of women and being the leading cause of preventable deaths during the perinatal and postpartum period, maternal mental health conditions are chronically understudied. This study is the first to identify spatial patterns in perinatal mental health conditions, and relate these patterns to place-based social and environmental factors that drive cluster development. Methods: We performed spatial clustering analysis of emergency department (ED) visits for perinatal mood and anxiety disorders (PMAD), severe mental illness (SMI), and maternal mental disorders of pregnancy (MDP) using the Poisson model in SatScan from 2016 to 2019 in North Carolina. Logistic regression was used to examine the association between patient and community-level factors and high-risk clusters. Results: The most significant spatial clustering for all three outcomes was concentrated in smaller urban areas in the western, central piedmont, and coastal plains regions of the state, with odds ratios greater than 3 for some cluster locations. Individual factors (e.g., age, race, ethnicity) and contextual factors (e.g., racial and socioeconomic segregation, urbanity) were associated with high risk clusters. Conclusions: Results provide important contextual and spatial information concerning at-risk populations with a high burden of maternal mental health disorders and can better inform targeted locations for the expansion of maternal mental health services.

10.
Int J Cardiol ; 363: 218-224, 2022 09 15.
Article En | MEDLINE | ID: mdl-35772579

BACKGROUND: Angiographic evidence of cardiac allograft vasculopathy (CAVangio) is a major limiting factor to survival after heart transplantation (HTx). Prevention of CAVangio is therefore most relevant. Whether modifiable risk factors could be targeted for the prevention of fibrotic plaques, that are common and related to CAVangio, is not clear. METHODS AND RESULTS: In a cohort of 74 consecutive HTx patients (median post-transplant interval 9.2 [4.1-15.5] years), we used the high resolution of optical coherence tomography (OCT) to quantify angulation parameters (maximal and mean arc) and plaque load (mean arc*relative plaque length) of fibrotic plaques. Mean arc was defined as the mean value of all angulation measurements per patient. We assessed the association between cardiovascular risk factors and OCT findings. Linear regression analysis showed a significant association of TG/HDL-c with mean fibrotic arc (12.7 [3.9-21.5], p = 0.006) and fibrotic plaque load (2298 [617-3979], p = 0.009) after adjustment for recipient age and sex. We used the median value of fibrotic plaque load to define high fibrotic plaque load. In binary logistic regression analysis, TG/HDL-c (odds ratio [OR] 1.81 with 95% CI [1.09-3.03], p = 0.02) and Lp(a) (OR 1.02 [1.00-1.05], p = 0.02) were associated with high fibrotic plaque load. Multivariable logistic regression analysis confirmed Lp(a) as significant predictor of high fibrotic plaque load (OR 1.03 [1.01-1.05], p = 0.02). CONCLUSION: TG/HDL-c ratio, a surrogate of insulin resistance syndrome, and Lp(a) were significantly associated with fibrotic plaque in HTx patients. Insulin resistance syndrome and Lp(a) might therefore represent additional targets for CAV prevention.


Coronary Artery Disease , Heart Transplantation , Insulin Resistance , Metabolic Syndrome , Plaque, Atherosclerotic , Allografts , Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/epidemiology , Fibrosis , Heart Transplantation/adverse effects , Humans , Metabolic Syndrome/complications , Plaque, Atherosclerotic/complications , Tomography, Optical Coherence/methods
11.
Int J Cardiol ; 358: 17-24, 2022 07 01.
Article En | MEDLINE | ID: mdl-35447273

BACKGROUND: Intimal hyperplasia in cardiac allograft vasculopathy (CAVIH) is limiting survival in pediatric and adult patients after heart transplantation (HTx). Analysis of risk factors for CAVIH using the high resolution of intracoronary optical coherence tomography (OCT) is scarce, particularly in children, and recommendations for CAV prevention are largely based on data obtained in adults. Whether the predictive value of risk factors is age- or sex-dependent is unknown. METHODS AND RESULTS: We used OCT to test the age- and sex-dependency of established risk factors regarding pathological CAVIH in a cohort of 102 pediatric and adult HTx patients (35% <18 years, 69% male). Modifiable parameters such as lipid values, and the diagnoses of dyslipidemia and diabetes showed age- and sex-dependent differences. Regarding CAVIH, receiver-operating characteristic analysis showed that LDL-c was relevant only in female patients (area under the curve [AUC] 0.79, p = 0.007), and total cholesterol in female (AUC 0.81; p = 0.004) and pediatric patients (AUC 0.73, p < 0.05). The association of dyslipidemia with CAVIH was stronger in adult (odds ratio [OR] 6.33) than in pediatric patients (OR 5.00) and in women (OR 6.00) than in men (OR 4.57). Diabetes was associated with CAVIH only in women (OR 11.25). CONCLUSION: In our cohort, modifiable risk factors, particularly total cholesterol and dyslipidemia, had a different impact depending on age and sex. Targeting risk factors in selected patients might improve individual CAVIH prevention.


Coronary Artery Disease , Heart Transplantation , Adult , Allografts , Child , Cholesterol , Coronary Artery Disease/etiology , Female , Heart Transplantation/adverse effects , Humans , Hyperplasia/etiology , Male , Risk Factors , Tomography, Optical Coherence/methods
12.
Int J Cardiol ; 356: 45-50, 2022 06 01.
Article En | MEDLINE | ID: mdl-35395286

OBJECTIVES: We analyzed the early and long-term survival after ABO-compatible heart transplantation in children under 3 years of age from 1991 to 2021 at our center. This retrospective and descriptive study aimed to identify serious adverse events associated with mortality after pediatric heart transplantation. PATIENTS AND METHODS: 46 patients with congenital heart failure (37%) in end-stage heart failure have undergone a pediatric heart transplantation. Primary outcome of interest was survival at follow-up time. RESULTS: Median (IQR) follow-up time (y), age (y), body-weight (kg) and BMI (kg/cm2) were 13.2 (5.7-19.5), 0.9 (0.2-2.0), 6.8 (4.3-10.0) and 14.2 (12.3-15.7). Twenty-four (52%) patients were male. 15 patients (33%) had a single ventricle physiology. At 30- days survival rate was 94 ± 4%. Survival rate at 1, 5, 10 and 15 years post HTx was 87 ± 5%, 84 ± 6%, 79 ± 6% and 63 ± 8%. One child underwent re-transplantation after 4 years, and another one after 11 years - in both cases due to graft failure. Higher early mortality in patients under 3 months of age and in patients with single ventricle physiology. Transplant free survival at 15 years was in children with cardiomyopathy better (71 ± 10%) than in those with congenital heart disease (50 ± 13%). One or more previous heart surgeries prior to HTx (n = 21) were associated to more mortality. CONCLUSION: Pediatric heart transplantation has acceptable long-term results and is still the best therapeutic option in children with end-stage cardiac failure. Underlying anomalies and single ventricle physiology, age below 3 months had a significant impact on survival.


Cardiomyopathies , Heart Defects, Congenital , Heart Failure , Heart Transplantation , Child , Child, Preschool , Female , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/surgery , Heart Failure/diagnosis , Heart Failure/surgery , Humans , Infant , Male , Retrospective Studies , Treatment Outcome
13.
Transplant Direct ; 8(1): e1266, 2022 Jan.
Article En | MEDLINE | ID: mdl-34966839

BACKGROUND: The development and progression of cardiac allograft vasculopathy documented by coronary angiography (CAVangio) after heart transplantation (HTx) has prognostic relevance. Yet there are limited data regarding the role of concomitant intracoronary imaging in the presence CAVangio. In particular, atherosclerotic plaques might represent a potential target for prevention, but their impact on stenosis is understudied. METHODS: We used high-resolution intracoronary optical coherence tomography (OCT) to quantify and compare findings of intimal hyperplasia (IH) and plaque morphologies in HTx patients (fibrotic plaque, lipid plaque, and calcified plaque). OCT findings were related to the presence of CAVangio as well as to the severity of stenosis. RESULTS: We included 65 consecutive patients into analysis (66% with CAVangio, posttransplant interval 9.9 ± 7.6 y). Fibrotic, lipid, and calcified plaques were present in 41 (63.1%), 39 (60%), and 18 (27.7%) patients, respectively. In addition to IH, the presence of fibrotic, lipid, and calcified plaques was found to be associated with CAVangio. The prevalence of lipid plaque and quantitative measurements of fibrotic plaque increased with stenosis severity (lipid plaque, P < 0.001, maximal and mean fibrotic arc, P = 0.05 and P = 0.001, respectively). Receiver operating characteristic analysis showed that area under the curve of the fibrotic plaque parameter mean fibrotic arc (0.87, 95% confidence interval [0.76-0.99]; P = 0.002) was superior to area under the curve of intima parameters regarding CAVangio. The effect of mean fibrotic arc (r = 0.52, P < 0.001) was relevant regarding stenosis severity. CONCLUSIONS: After a longer posttransplant interval, CAV findings in OCT included a combination of IH and atherosclerotic plaques. In addition to IH, the presence of fibrotic, lipid, and calcified plaques is associated with CAVangio. Further studies are warranted to evaluate if the in vivo screening for plaque progress, particularly of fibrotic plaque, could improve individual secondary prevention and outcome in HTx patients.

14.
Transpl Int ; 34(12): 2578-2588, 2021 12.
Article En | MEDLINE | ID: mdl-34709681

Reduced adherence after heart transplantation increases the risk for acute rejection. Therefore, the aim of this study was to evaluate the patient's satisfaction with outpatient care and quality of life (QOL) after pediatric and adult heart transplantation. Observational study after pediatric (n = 22) and adult (n = 65) heart transplantation and the parents of the pediatric patients (n = 22) to evaluate the patients' satisfaction with outpatient care and QOL. Established standardized questionnaires were used for patient satisfaction (ZAP survey) and QOL (SF36); the latter was compared with the cohort of the BGS98 survey (BGS98 cohort). ZAP score: excellent results with almost all values >80. QOL: pediatric cohort showed significantly higher values in physical functioning (P = 0.041) and role physical (P = 0.003) but significantly lower values in the sub-scale general health (P = 0.02) compared to adult cohort. In comparison with BGS98 cohort, children showed almost similar results, whereas adult cohort showed worse values in physical and emotional functioning, but higher values regarding general health. The QOL of patients after pediatric heart transplantation is comparable to a standardized reference population in Germany, whereas adult patients show reduced physical and emotional functioning, but better values regarding general health. The patients' satisfaction with the outpatient care is very high.


Heart Transplantation , Quality of Life , Adult , Ambulatory Care , Child , Humans , Patient Satisfaction , Surveys and Questionnaires
15.
Mol Genet Metab Rep ; 28: 100776, 2021 Sep.
Article En | MEDLINE | ID: mdl-34178604

Carnitine transporter defect (CTD) is a potentially life-threatening disorder causing acute metabolic decompensation, cardiac arrhythmia, and cardiac and skeletal myopathies. CTD is included in many newborn screening (NBS) programs. The screening parameter free carnitine, however, is influenced by maternal conditions due to placental transfer. This study reviewed the NBS results for CTD as part of a pilot study in Bavaria, Germany, and the long-term follow-up of the identified patients treated in our center between January 1999 and June 2018. Among 1,816,000 Bavarian NBS samples, six newborns were diagnosed with CTD (incidence of 1:302,667; positive predictive value (PPV) of 1.63% from 2008 to 2018). In the 24 newborns presented to our center for confirmatory testing, we detected four newborns and six mothers with CTD, one newborn and three mothers in whom CTD was presumed but not genetically confirmed, and one mother with glutaric aciduria type I. In 11 newborns, no indication for an inborn error of metabolism was found. The newborns and mothers with CTD had no serious cardiac adverse events or relevant muscular symptoms at diagnosis and during treatment for up to 14 years. Three mothers were lost to follow-up. Revealing a lower incidence than expected, our data confirm that NBS for CTD most likely misses newborns with CTD. It rather produces high numbers of false-positives and a low PPV picking up asymptomatic mothers with a diagnosis of uncertain clinical significance. Our data add to the growing evidence that argues against an implementation of CTD in NBS programs.

16.
Int J Cardiol ; 328: 227-234, 2021 04 01.
Article En | MEDLINE | ID: mdl-33316256

BACKGROUND: Intracoronary imaging enables an early detection of intimal changes. To what extend the development of absolute and relative intimal hyperplasia in intracoronary imaging differs depending on age and post-transplant time is not known. METHODS: Aim of our retrospective study was to compare findings between 24 pediatric (cohort P) and 21 adult HTx patients (cohort A) using optical coherence tomography (OCT) at corresponding post-transplant intervals (≤5 years: P1 (n = 11) and A1 (n = 10); >5 and ≤ 10 years: P2 (n = 13) and A2 (n = 11),. Coronary intima thickness (IT), media thickness (MT) and intima to media ratio (I/M) were assessed per quadrant. Maximal IT >0.3 mm was considered absolute, I/M > 1 relative intimal hyperplasia. RESULTS: Compared to A1, I/M was significantly higher in P1 (maximal I/M: P1: 5.41 [2.81-13.39] vs. A1: 2.30 [1.55-3.62], p = 0.005), whereas absolute IT values were comparable. In contrast, I/M was comparable between P2 and A2, but absolute IT were significantly higher in A2 (maximal IT: P2: 0.16 mm [0.11-0.25] vs. A2: 0.40 mm [0.30-0.71], p < 0.001). A2 presented with higher absolute IT (maximal: A1: 0.16 mm [0.12-0.44] vs. A2: 0.40 mm [0.30-0.71], p = 0.02) and I/M (maximal I/M A1: 2.30 [1.55-3.62] vs. A2: 3.79 [3.01-5.62], p = 0.04). CONCLUSION: Our results suggest an age- and time-dependent difference in the prevalence of absolute and relative intimal hyperplasia in OCT, with an early peak in children and a progressive increase in adults.


Coronary Artery Disease , Heart Transplantation , Adult , Allografts , Child , Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/pathology , Coronary Vessels/diagnostic imaging , Coronary Vessels/pathology , Heart Transplantation/adverse effects , Humans , Hyperplasia/diagnostic imaging , Hyperplasia/pathology , Retrospective Studies , Tomography, Optical Coherence
17.
Clin Transplant ; 35(3): e14191, 2021 03.
Article En | MEDLINE | ID: mdl-33315277

BACKGROUND: Cardiac allograft vasculopathy (CAV) and nephrotoxicity affect long-term survival after heart transplantation (HTX). Studies, mostly conducted in adults, showed a positive effect of everolimus (EVL) on these problems. We describe the effects of conversion of the immunosuppressive therapy to an everolimus including regime on CAV, renal function, and safety in heart transplanted children/adolescents. METHODS: This retrospective single-center study included 36 participants (mean time after HTX 6.3 ± 4.7 years). Descriptive pre/post-comparisons were performed with an observation period partially up to 4 years. Impact on CAV was assessed based on intravascular imaging and Stanford grading. Safety analysis included cytomegalovirus (CMV)-infection and acute rejection. RESULTS: In terms of CAV (9 out of 36 patients) four showed no progression, three an improvement, one a worsening; one new diagnosis. The average CrCl showed a significant improvement 6, 12, and 24 months after conversion regarding all patients (n = 29). There was no acute rejection or CMV-infection. CONCLUSION: Conversion to an EVL-based therapy after pediatric HTX is a safe immunosuppressive regime without increasing risk of acute rejection or CMV-infection. There was some evidence of reduction in progression of CAV and a significant improvement of the renal function.


Everolimus , Heart Transplantation , Adolescent , Adult , Allografts , Child , Everolimus/therapeutic use , Graft Rejection/drug therapy , Graft Rejection/etiology , Graft Rejection/prevention & control , Heart Transplantation/adverse effects , Humans , Immunosuppressive Agents/therapeutic use , Retrospective Studies
18.
Int J Cardiol ; 325: 45-50, 2021 02 15.
Article En | MEDLINE | ID: mdl-33049296

BACKGROUND: Conduit dilatation above 110% and TPVI in conduits <16 mm is not recommended. However, if we want to reach normal values for RVOT diameters and diminish reintervention rates, pushing these boundaries is essential. METHODS: Analysis of subsequent patients who underwent TPVI with Edwards Sapien valves in conduits ≤16 mm between 2010 and 2020. RESULTS: In n = 33 cases median age was 13 years (5-20 y) and median weight 47 kg (15-91 kg). Preexisting RVOT grafts were n = 28 Contegra® conduits and n = 5 homografts (12 mm n = 15; 14 mm n = 11; 16 mm n = 7). Implanted were the Sapien (n = 8), Sapien XT (n = 10) and Sapien 3 valve (n = 15) with 20 mm (n = 4), 23 mm (n = 19), 26 mm (n = 9) and 29 mm (n = 1). Mean minimal RVOT diameter after TPVI was 22,7 ± 2,3 mm (18-30 mm) which is 150% of the mean minimal RVOT diameter before TPVI (15,1 ± 4,3 mm). Covered stents were used in n = 10 cases. Contained conduit rupture occurred in n = 7 cases (21%). Residual RVOT gradients of 5,7 ± 4,9 mmHg (0-18 mmHg) showed adequate RV unloading. CONCLUSION: TPVI could be performed successfully in all patients. Dilatation above 150% and a valve/conduit diameter ratio up to 2,4 were well tolerated. There was a considerable amount of conduit rupture but all were confined without further need for intervention or surgery.


Heart Valve Prosthesis Implantation , Heart Valve Prosthesis , Pulmonary Valve Insufficiency , Pulmonary Valve , Adolescent , Cardiac Catheterization , Feasibility Studies , Humans , Prosthesis Design , Pulmonary Valve/diagnostic imaging , Pulmonary Valve/surgery , Pulmonary Valve Insufficiency/surgery , Retrospective Studies , Treatment Outcome
19.
J Trop Pediatr ; 66(2): 187-193, 2020 04 01.
Article En | MEDLINE | ID: mdl-31377805

BACKGROUND: Burden and pattern of congenital anomalies are insufficiently reported in Vietnam. This study aims to determine the prevalence and pattern of congenital anomalies in neonates in a tertiary hospital in central Vietnam. METHODS: A prospective cross-sectional study recruited all newborns with congenital anomalies in Da Nang Hospital for Women and Children-where nearly 60% neonates in the city are delivered. RESULTS: Over a 1-year period, 551 out of 14 335 registered live births were found to have congenital anomalies, equivalent to an overall prevalence of 384.4 per 10 000 live births. Congenital heart defects were the most common type (52.3%) with the prevalence of 200.9 per 10 000 live births, followed by anomalies of musculoskeletal system, digestive system. CONCLUSIONS: This study revealed a high prevalence of congenital anomalies with the most common type being congenital heart defects in central Vietnam compared to both higher income countries and resource-limited settings.


Congenital Abnormalities/epidemiology , Heart Defects, Congenital/epidemiology , Adult , Cross-Sectional Studies , Female , Humans , Infant, Newborn , Male , Prevalence , Prospective Studies , Tertiary Care Centers , Vietnam/epidemiology
20.
World J Pediatr Congenit Heart Surg ; 11(4): NP37-NP40, 2020 Jul.
Article En | MEDLINE | ID: mdl-28673109

Takotsubo cardiomyopathy describes a sudden onset cardiomyopathy with acute impairment of left ventricular function and spontaneous resolution over time. Only a few cases of Takotsubo cardiomyopathy in childhood have been described. We report the case of a 12-year-old girl with repaired tetralogy of Fallot who experienced acute onset of left ventricular dysfunction without coronary arterial involvement, suggesting Takotsubo cardiomyopathy, during an interventional catheterization procedure. Cardiogenic shock necessitated mechanical circulatory support with extracorporeal membrane oxygenator for ten days and mechanical ventilation for 12 days. The girl recovered without sequelae. Percutaneous pulmonary valve implantation was performed four months later without complications. Unusual aspects of this case include the use of mechanical circulatory support during the recovery phase of Takotsubo cardiomyopathy in a patient with congenital heart disease.


Heart Valve Prosthesis Implantation/adverse effects , Postoperative Complications , Pulmonary Valve Insufficiency/surgery , Pulmonary Valve/surgery , Takotsubo Cardiomyopathy/etiology , Ventricular Function, Left/physiology , Cardiac Catheterization , Child , Echocardiography , Electrocardiography , Female , Humans , Magnetic Resonance Imaging, Cine , Pulmonary Valve Insufficiency/diagnosis , Takotsubo Cardiomyopathy/diagnosis , Takotsubo Cardiomyopathy/physiopathology
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